Highlights
- •In general, pediatric ADS are more often associated with a monophasic course and longer relapse-free period.
- •Laboratory, electrophysiological and neuroimaging examinations contribute to the recognition of ADS phenotype but only monitoring the clinical course and regular control of these examinations would increase the yield of such investigations.
- •Contrary to ADEM, MS and NMOSD-patients, RD-NOS-patients showed misleading intermediate phenotype with common clinical features with MS and ADEM and overlapping neuroimaging features with ADEM and NMOSD.
Abstract
Introduction
The yearly incidence of Acute Demyelinating Syndromes (ADS) in a multiethnic cohort
of children published by Langer-Gould and al in 2011 was estimated at about 1.66 per
100,000. Nevertheless, the real incidence for these disorders is still underestimated
as the iterative revision for diagnosis criteria have failed to classify a significant
number of children with ADS.
Purpose
This work was aimed to describe clinical and paraclinical characteristics of ADS in
a pediatric population.
Material and methods
Demographic, clinical and paraclinical data of 42 children (24 females; 18 male; SR = 1.33),
were collected from the medical records of patients admitted to the child neurology
department of Sfax University Hospital between 2008 and 2021 for clinical events with
presumed inflammatory origin. Next, patients were categorized as per M. N. Nouri and
al. up dated classification for ADS. Finally, characteristics of different ADS categories
were compared.
Results
The mean age onset was 6 years (± 3.5 years). For a mean follow-up period of 28 months,
69% of patients had a monophasic course. ADS in our pediatric population were Acute
disseminated encephalomyelitis (ADEM) (36%), Clinically isolated syndrome (CIS) (24%),
Multiple sclerosis (MS) (19%), Neuromyelitis optica spectrum disorder (NMOSD) (7%),
Myelin oligodendrocyte glycoprotein antibodies-associated diseases (MOGAD) (2%) and
Recurrent demyelinating disease not otherwise specified (RD-NOS) (10%). At presentation,
patients showed different clinical picture according to ADS-subtype with more patients
with epileptic seizure in ADEM-group (53.3%), optic neuritis in CIS-group (70%), motor
deficit in MS-group (62.5%), area postrema syndrome in NMOSD-group (33.3%) and vesico-sphincter
dysfunction in RD-NOS-group (75%). Among patients presenting with visual impairment
(21.4%), Visual evoked potential (VEP) guided the diagnosis of NMOSD in 22.2% by objectifying
axonal optic nerve damage. Different ADS subtypes were identified according to MRI
results in 100% of ADEM-patients and 75% of MS-patients and on antibody testing in
three patients. The ADS-subtype was recognized based on antibody testing in three
patients. Two patients from CIS-group: the first with isolated optic neuritis (ON)
was positive for antiaquaporin 4 antibodies (anti-AQP4) and the other with clinically
polyfocal ADS was positive for antinuclear antibodies (ANA) type anti-RNP. The remaining
patients who presented with ADEM-phenotype was positive for anti-myelin oligodendrocyte
glycoprotein (anti-MOG).
Significance
Recognizing distinctive features of each ADS category may improve diagnosis accuracy
as well as the indication of suitable treatment.
Keywords
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Article info
Publication history
Published online: September 27, 2021
Accepted:
September 27,
2021
Received in revised form:
September 26,
2021
Received:
July 30,
2021
Identification
Copyright
© 2021 Elsevier B.V. All rights reserved.
